• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.24-33
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• 2016

Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

• The Journal of Korean Society for Radiation Therapy
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• v.28 no.2
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• pp.179-186
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• 2016

Purpose : To figure out if the treatment plan for rectum, bladder and prostate that have a lot of interfraction errors satisfies dosimetric limits without adaptive plan by analyzing MR image. Materials and Methods : This study was based on 5 prostate cancer patients who had IMRT(total dose: 70Gy) Using ViewRay MRIdian System(ViewRay, ViewRay Inc., Cleveland, OH, USA) The treatment plans were made on the same CT images to compare with the plan quality according to adaptive plan, and the Eclipse(Ver 10.0.42, Varian, USA) was used. After registrate the 5 treatment MR images to the CT images for treatment plan to analyze the interfraction changes of organ, we measured the dose volume histogram and the changes of the absolute volume for each organ by appling the first treatment plan to each image. Over 5 fractions, the total dose for PTV was $V_{36.25}$ Gy $${\geq_-}$$ 95%. To confirm that the prescription dose satisfies the SBRT dose limit for prostate, we measured $V_{100%}$, $V_{95%}$, $V_{90%}$ for CTV and $V_{100%}$, $V_{90%}$, $V_{80%}$ $V_{50%}$ of rectum and bladder. Results : All dose average value of CTV, rectum and bladder satisfied dose limit, but there was a case that exceeded dose limit more than one after analyzing the each image of treatment. After measuring the changes of absolute volume comparing the MR image of the first treatment plan with the one of the interfraction treatment, the difference values were maximum 1.72 times at rectum and maximum 2.0 times at bladder. In case of rectum, the expected values were planned under the dose limit, on average, $V_{100%}=0.32%$, $V_{90%}=3.33%$, $V_{80%}=7.71%$, $V_{50%}=23.55%$ in the first treatment plan. In case of rectum, the average of absolute volume in first plan was 117.9 cc. However, the average of really treated volume was 79.2 cc. In case of CTV, the 100% prescription dose area didn't satisfy even though the margin for PTV was 5 mm because of the variation of rectal and bladder volume. Conclusion : There was no case that the value from average of five fractions is over the dosimetric limits. However, dosimetric errors of rectum and bladder in each fraction was significant. Therefore, the precise delivery is needed in case of prostate SBRT. The real-time tracking and adaptive plan is necessary to meet the precision delivery.

• Journal of the HCI Society of Korea
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• v.8 no.1
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• pp.1-9
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• 2013

This is to develop a new way of testing self-esteem by adopting DAP(Draw a Person) test and to make a platform to digitalize it for young people in the adolescent stage. This approach is to get high effectiveness of the self-esteem measurement using DAP test, including some personal inner situations which can be easily missed in the large statistical analysis. The other objective of this study is digitalize to recover limits of DAP test in the subjective rating standard. It is based on the distribution of the figure drawing expressed numerically by the anxiety index of Handler. For these two examinations, we made experiment through 4 stages with second grade middle school 73 students from July 30th to October 31th in 2009 during 4 months. Firstly, we executed 'Self Values Test' for all 73 people, and divided them into two groups; one is high self-esteem group of 36 people, the other is low self-esteem group of 37 people. Secondly, we regrouped them following D (Depression), Pd (Psychopathic Deviate), Sc (Schizophrenia) scales of MMPI; one is high self-esteem group of 7 people, the other is low self-esteem group of 13 people. Thirdly, we conducted DAP test separately for these 20 people. We intended to verify necessity and appropriateness of direction of 'Digitalizing Measurement System' by comparing and analyzing relation between DAP and Self-esteem following evaluation criteria which has similarity in 3 tests, after executing DAP to reflect peculiarity of adolescents sufficiently. We compared and analyzed result abstracted by sampling DAP test of two groups; One is high self-esteem group of 2 people, the other is low self-esteem group of 2 people; to confirm whether we can improve limitation that original psychological testing has by comparing mutual reliance of measurement test. Finally, with DAP test gained from correlations between self-esteem and melancholia following as above-mentioned steps, we discovered possibility of realization to get a concrete and individual criteria of evaluation based on Expert System as a way of enhancing accessibility in quantitative manner. 'Digitalizing Measurement Program' of DAP test suggested in this study promote results' reliability based on existing tests and measurement.

• 한국유가공학회:학술대회논문집
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• 2007.09a
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• pp.49-58
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• 2007

Inflammatory process leads to the well-known mucosal damage and therefore a further disturbance of the epithelial barrier function, resulting abnormal intestinal wall function, even further accelerating the inflammatory process[1]. Despite of the records, etiology and pathogenesis of IBD remain rather unclear. There are many studies over the past couple of years have led to great advanced in understanding the inflammatory bowel disease(IBD) and their underlying pathophysiologic mechanisms. From the current understanding, it is likely that chronic inflammation in IBD is due to aggressive cellular immune responses including increased serum concentrations of different cytokines. Therefore, targeted molecules can be specifically eliminated in their expression directly on the transcriptional level. Interesting therapeutic trials are expected against adhesion molecules and pro-inflammatory cytokines such as TNF-${\alpha}$. The future development of immune therapies in IBD therefore holds great promises for better treatment modalities of IBD but will also open important new insights into a further understanding of inflammation pathophysiology. Treatment of cytokine inhibitors such as Immunex(Enbrel) and J&J/Centocor(Remicade) which are mouse-derived monoclonal antibodies have been shown in several studies to modulate the symptoms of patients, however, theses TNF inhibitors also have an adverse effect immune-related problems and also are costly and must be administered by injection. Because of the eventual development of unwanted side effects, these two products are used in only a select patient population. The present study was performed to elucidate the ability of TNF-${\alpha}$ antibodies produced in sheep colostrums to neutralize TNF-${\alpha}$ action in a cell-based bioassay and in a small animal model of intestinal inflammation. In vitro study, inhibitory effect of anti-TNF-${\alpha}$ antibody from the sheep was determined by cell bioassay. The antibody from the sheep at 1 in 10,000 dilution was able to completely inhibit TNF-${\alpha}$ activity in the cell bioassay. The antibodies from the same sheep, but different milkings, exhibited some variability in inhibition of TNF-${\alpha}$ activity, but were all greater than the control sample. In vivo study, the degree of inflammation was severe to experiment, despite of the initial pilot trial, main trial 1 was unable to figure out of any effect of antibody to reduce the impact of PAF and LPS. Main rat trial 2 resulted no significant symptoms like characteristic acute diarrhea and weight loss of colitis. This study suggested that colostrums from sheep immunized against TNF-${\alpha}$ significantly inhibited TNF-${\alpha}$ bioactivity in the cell based assay. And the higher than anticipated variability in the two animal models precluded assessment of the ability of antibody to prevent TNF-${\alpha}$ induced intestinal damage in the intact animal. Further study will require to find out an alternative animal model, which is more acceptable to test anti-TNF-${\alpha}$ IgA therapy for reducing the impact of inflammation on gut dysfunction. And subsequent pre-clinical and clinical testing also need generation of more antibody as current supplies are low.